Regular Blood Tests
Visits to the PKU Clinic
Keeping a Food Diary
PKU Lessons for Your Child’s Teacher
Sick Days and PKU
PKU is a lifelong (chronic) condition. That means your baby will need to have frequent
blood tests throughout his or her life. Blood tests are done at a PKU clinic and
at home, sometimes as often as weekly.
These blood tests measure Phe levels. Phe is an amino acid found in food. Amino
acids are the building blocks of protein. Measuring Phe levels is essential for
keeping PKU under control.
Learn more about Phe levels.
Your doctor and dietitian use these blood tests to adjust the special milk-free
formula your baby needs during his or her early development. Later, as your child
grows, these blood tests will be used to make adjustments in the
Phe-free medical
foods and Phe-restricted diet to ensure adequate Phe control and nutritional balance.
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Regular visits to a PKU clinic are important to your child’s normal development.
Think of the clinic as your partner in helping you manage your child’s PKU.
Find a PKU clinic in your area.
What you’ll find at the PKU clinic
- Dietitians to help you recognize which foods are low and high in Phe. They
will help determine the amount of Phe your child can handle. This is called Phe
tolerance.
You will learn to keep a food diary to track the amount of Phe your child consumes
each day. Dietitians may assist you with menu planning as your child’s nutritional
needs change.
Depending on your child’s age and treatment goals, the dietitian, in consultation
with your doctor, may suggest adding KUVAN to the Phe-restricted diet. KUVAN may
help lower Phe in your child.
Learn more about KUVAN.
- Educational classes geared toward specific age groups for children. The
classes help your child identify foods high and low in Phe.
As your child grows, he or she will learn to take an active role in choosing and
even helping to prepare low-Phe meals. Such participation increases the likelihood
that your child will stick to the Phe-restricted diet.
- Doctors who measure your child’s physical and mental growth and prescribe
treatment for PKU.
- Genetic counselors who educate and support patients and their families
regarding inherited diseases.
- Nurses who may take vital signs, interview you about your health since
your last visit, draw your blood, and help coordinate your care.
- Support from other parents who have children with PKU. You will become
part of the PKU community, where you can share your experiences with others like
you.
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A food diary helps your dietitian see how much Phe your child consumes over several
days. The dietitian uses that information to adjust the Phe-restricted diet and
Phe-free medical foods as necessary. So it’s very important to accurately record
what—and how much—your child eats and drinks each day.
The sample food diary below shows one day’s menu for a Phe-restricted diet. Note
that your child’s diet may differ from the one shown here.
You can also download a 3-day diet record to write down your child’s food
and beverage intake, which is a dependable way to determine your child’s Phe intake.
Sample Phe-restricted menu (1 day’s menu)
Use the blank spaces to fill in your child’s own daily menu.
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Help your child’s teacher understand PKU. Download a brochure that
provides important information that a teacher, babysitter, or other kind of caretaker
needs to know about PKU and the Phe-restricted diet before watching over your child.
Once your child enters school, sticking to a Phe-restricted diet may be more difficult
for him or her. Trading that apple for a PB&J sandwich is very tempting. That’s
where your child’s teacher can help.
The teacher plays an important role in reinforcing your child’s diet. The teacher
can:
- Notify you of class parties where food will be served.
- Make sure that your child takes home all uneaten food from lunch so they can be recorded
in the food diary.
- Enforce a class policy against food trading.
- Make sure your child eats the food you provided.
Give your child’s teacher a note as a friendly reminder about your child’s special
dietary needs. Together, you and your child’s teacher can help keep your child on
the Phe-restricted diet with as little cheating as possible. Click on the image
to download.
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What should you do when your child gets sick?
- While Phe levels are likely to rise during the illness, this elevation is most likely
temporary.
- Treat the illness first.
- Your child may or may not have an appetite. However, don’t force your child to eat.
- Keep your child hydrated. Water, fruit juice, low-protein gelatin, and low-protein
broth are good choices.
- Ease your child back into the Phe-restricted diet. It may take several days before
your child can resume the Phe-restricted diet.
- Ask your doctor or dietitian what other steps you should take during your child’s
illness.
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Indication
KUVAN® (sapropterin dihydrochloride) Tablets is approved to reduce blood Phe levels
in patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-)
responsive Phenylketonuria (PKU). KUVAN is to be used with a Phe-restricted diet.
Important Safety Information
High blood Phe levels are toxic to the brain and can lead to lower intelligence
and decrease in the ability to focus, remember and organize information. Any change
you make to your diet may impact your blood Phe level. Follow your doctor’s instructions
carefully. Your doctor and dietitian will continue to monitor and may adjust your
diet throughout your treatment with KUVAN.
If you have a fever, or if you are sick, your Phe level may go up. Tell your doctor
and dietitian as soon as possible so they can see if they have to adjust your treatment
to help keep your blood Phe levels in the desired range.
KUVAN is a prescription medicine and should not be taken by people who are allergic
to any of its ingredients. Tell your doctor if you have ever had liver or kidney
problems, are nursing or pregnant or may become pregnant, have poor nutrition or
are anorexic. Your doctor will decide if KUVAN is right for you. Tell your doctor
about all the medicines you take.
The most common side effects reported when using KUVAN are headache, diarrhea, abdominal
pain, upper respiratory tract infection (like a cold), throat pain, vomiting, and
nausea.
To report SUSPECTED ADVERSE REACTIONS, contact BioMarin Pharmaceutical Inc. at 1-866-906-6100, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
Please read the full Patient Information by clicking here.